His plane was preparing to land at Philadelphia International Airport when Allen M. Weiss, a marketing professor at the University of Southern California, felt a spasm of pain in his left cheek, near his nose. “It was really strange,” recalled Weiss, then director of Mindful USC, a group of meditation-based programs at the University of Los Angeles. “My face froze.”
Within minutes, the pain disappeared and the last leg of Weiss’ trip home to California in December 2015 was uneventful. But during the following months the sensation reappeared in the same place. At first, the unpredictable pain was quite mild and simply annoying; later it became an unbearable daily torment.
Several years after the pain first appeared, Weiss, who had consulted dentists, oral pain experts, and an otolaryngologist, received a diagnosis that turned out to be correct. But his complicated medical history, a radiology report that failed to describe an important finding, and a cryptic warning from one of his doctors delayed effective treatment for three more years.
“It was completely confusing,” Weiss said. In June 2023 he underwent surgery that has significantly reduced his pain and improved his quality of life.
N Nicole Moayeri, a neurosurgeon in Santa Barbara, California, who operated on Weiss, said a prolonged search for a diagnosis and treatment is not unusual for those suffering from the rare Weiss disease.
“I commonly see people who have had multiple dental procedures over the years” when the problem was not in the mouth, Moayeri said. “It’s really shocking to me that so many people suffer” from this for so long.
After three months of intermittent pain after the flight, Weiss consulted her internist. For reasons that are unclear, the doctor told Weiss that the cause was probably psychological, not physical, and that it was not serious.
She referred Weiss to an ear, nose and throat specialist whom she saw in March 2016. She performed an exam and ordered a CT scan that revealed a deviated septum, a typically painless condition estimated to affect up to 80 percent of people. population in which the The bone or cartilage that divides the nasal passages is off-center. A moderate or severe deviation can contribute to the development of sinus infections, headaches, and breathing problems. But Weiss was having none of that. And a deviated septum didn’t explain the pain spasms.
Weiss then consulted her dentist. She found nothing and referred Weiss to a colleague who specializes in treating oral pain. The specialist advised Weiss to open and close her mouth repeatedly while he sprayed the problem area with cold water.
“The idea was to train my mind not to pay attention to my pain,” Weiss said. He was also prescribed nortriptyline, an antidepressant also used to treat facial pain. None of the treatments were effective.
Trigger point injections
A few months later, Weiss consulted a second oral pain specialist, who recommended trigger point injections, anesthetic injections that are supposed to relieve muscle knots. Weiss said he received these injections every two weeks for the next few years. He also tried acupuncture.
“It’s really shocking to me that so many people suffer” from this problem.
The reason for the injections and acupuncture, which seemed to work only briefly, was unclear. At the time, “I didn’t ask any questions,” said Weiss, now 73. “I was just listening to my doctors: They’re some of the best in Los Angeles.”
But in late 2019, the cost of biweekly trigger point injections “was getting out of hand,” Weiss said; His health insurance covered only part of the fee. He decided to consult a neurologist.
The neurologist who examined him in January 2020 paid special attention to where the pain was concentrated on his face. He diagnosed trigeminal neuralgia (TN), a rare form of nerve pain that affects the trigeminal nerve, a cranial nerve that carries signals from the brain to the face. The intensity of pain caused by TN varies, but it can be so physically and mentally disabling that it has been nicknamed “the suicide disease.”
TN, which usually affects only one side of the face, is more common in women and people over 50 years of age and is often confused with a dental or jaw problem; It is estimated that between 10,000 and 15,000 cases are diagnosed annually. TN can be caused by a blood vessel pressing on the nerve or by an injury caused by sinus surgery or dental work. In some cases no cause is found. Sometimes people with multiple sclerosis develop TN because the disease destroys the myelin sheath that protects the nerves.
I am very glad I had the surgery, but I wish from the beginning I had gone online and started reading about facial pain.
-Allen M Weiss
First-line treatment includes medications. Surgery is reserved for cases where medications have failed to relieve pain.
The neurologist ordered a new series of injections and sent Weiss to a second neurologist who performed electromyography (EMG); The test ruled out neuromuscular disorders, including MS. Weiss said the neurologist advised him to “try every pain medication possible before even contemplating any surgery” for TN; Weiss didn’t ask why.
In early 2021, Weiss retired and moved north to Santa Barbara.
His pain had intensified to the point that he could not meditate, a daily practice he had begun 15 years earlier and taught. “It was very disturbing. This was a central part of my life,” she said.
During the pandemic, Weiss had put off dental care, so she made an appointment with a dentist in Santa Barbara. The experience proved to be excruciating, requiring multiple injections of novocaine while the dentist replaced a crown. Weiss said she “had the worst night of my life” trying in vain to sleep with ice packs on her face to dull the waves of spasmodic pain.
He had also begun to see a new round of specialists in Santa Barbara. Some wondered if TN was the cause of her pain and suggested that the problem could originate in her sinuses. Others were wary of additional brain surgery (the potential next step if medications failed) because of her medical history.
Pituitary adenoma, a benign brain tumor
In 1997, Weiss underwent surgery to remove a pituitary adenoma, a benign brain tumor that can cause hormonal imbalances. A year later he underwent radiation to eradicate possible residual disease. Several doctors seemed reluctant to recommend another operation that would affect his brain.
In June 2021, a neurologist, the third one he consulted, prescribed a new drug to treat nerve pain. He also ordered scans, including an MRI, to determine if there were abnormalities of the cranial nerves, particularly the trigeminal nerves. She told Weiss that the pain caused by NT was usually triggered by drinking cold water, eating, talking, or brushing her teeth, none of which seemed to bother her. Weiss’s pain tended to be positional: it got considerably worse when she lay down.
The MRI, performed in July 2021, did not find any abnormalities affecting Weiss’s trigeminal nerves. A CT scan indicated possible obstruction of the sphenoid sinuses, located at the back of the nose.
In early 2023, Weiss saw a new ENT doctor who told him he wasn’t sure what was wrong with him.
A new approach
Weiss, no closer to relief after more than seven years, said he felt hopeless and depressed.
“I decided I had to take control of the situation,” he recalled. She scheduled an appointment with another otolaryngologist and obtained records from her pituitary surgery and follow-up scans. She hoped they could help doctors determine whether she had a sinus problem or whether the finding on her 2021 CT scan reflected scar tissue from her 1997 operation, as one doctor had suggested.
After another false start (the new otolaryngologist detected a possible dental problem, prompting another visit to a dentist who found nothing), Weiss was referred to Moayeri, medical director of neurosurgical oncology at Cottage Health.
At their first meeting in May, the neurosurgeon said she noticed the focus on Weiss’ previous pituitary surgery and his sinuses, which “led him down the wrong path for a while…he’s been seeing a lot of doctors.”
He went back to the TN diagnosis and told Weiss he needed to review his scans. Moayeri also changed his medication to the anti-seizure drug known to be most effective in treating TN, which relieved some of his current pain.
“I wish from the beginning I had gone online and started reading about facial pain.”
When the neurosurgeon inspected the images from her 2021 MRI, she was surprised by what he saw, which disagreed with the radiologist’s conclusion that the trigeminal nerves were “normal.” The superior petrosal vein, she said, was compressing Weiss’s left trigeminal nerve.
“There is a lot of variability among medical professionals,” Moayeri said of the discrepancy.
Moayeri suspected the compressed nerve was the cause of his TN. A gentle brain operation called microvascular decompression can relieve pressure on the nerve by moving it away from the vein or blood vessels, compressing or irritating it, and protecting the nerve with a small Teflon pad.
Moayeri told Weiss that he was a candidate for decompression surgery, but that his atypical presentation of TN decreased the success rate, possibly by as much as 30 percent. And he listed the risks, including stroke, permanent facial numbness, worsening pain and infection.
To her surprise, Weiss didn’t hesitate: she wanted surgery.
“Nothing else had helped,” he said. “I was in pain every day and I thought I would be in increasing pain for the rest of my life. I thought it was my only option.”
Weiss said his pain has decreased considerably.
During the June 28 procedure, Moayeri discovered that smaller veins that drain into the petrosal vein were intertwined with the nerve, immobilizing and scarring it, making the painstaking surgery more difficult. As many years had passed since the time of his first attack, the neurosurgeon told Weiss that, although his severe pain might be reduced, the long delay meant that he might not disappear completely.
His prediction turned out to be correct. Weiss said his pain has decreased considerably, but he now feels intermittent pressure near his ear. Doctors have told him this may be the result of nerve scarring and could be permanent.
“I’m very glad I had the surgery,” he said, “but I wish from the beginning I had gone online and started reading about facial pain.” Weiss added that he believes he trusted doctors “too much” and “didn’t understand how limited” they were in his experience.
He said he especially regrets the years he wasted receiving expensive trigger point injections. “If I had found a doctor years ago who would order an MRI and send it to a neurosurgeon,” Weiss said, the compressed nerve “could have been caught sooner. And it could have prevented some of the nerve damage and pain.”